Maladaptive myelination promotes generalized epilepsy progression

Nat Neurosci. 2022 May;25(5):596-606. doi: 10.1038/s41593-022-01052-2. Epub 2022 May 2.

Abstract

Activity-dependent myelination can fine-tune neural network dynamics. Conversely, aberrant neuronal activity, as occurs in disorders of recurrent seizures (epilepsy), could promote maladaptive myelination, contributing to pathogenesis. In this study, we tested the hypothesis that activity-dependent myelination resulting from absence seizures, which manifest as frequent behavioral arrests with generalized electroencephalography (EEG) spike-wave discharges, promote thalamocortical network hypersynchrony and contribute to epilepsy progression. We found increased oligodendrogenesis and myelination specifically within the seizure network in two models of generalized epilepsy with absence seizures (Wag/Rij rats and Scn8a+/mut mice), evident only after epilepsy onset. Aberrant myelination was prevented by pharmacological seizure inhibition in Wag/Rij rats. Blocking activity-dependent myelination decreased seizure burden over time and reduced ictal synchrony as assessed by EEG coherence. These findings indicate that activity-dependent myelination driven by absence seizures contributes to epilepsy progression; maladaptive myelination may be pathogenic in some forms of epilepsy and other neurological diseases.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Disease Models, Animal
  • Electroencephalography
  • Epilepsy, Absence*
  • Epilepsy, Generalized* / genetics
  • Mice
  • NAV1.6 Voltage-Gated Sodium Channel
  • Rats
  • Rats, Wistar
  • Seizures

Substances

  • NAV1.6 Voltage-Gated Sodium Channel
  • Scn8a protein, mouse